Clinical characteristics of high-grade B-cell lymphomas with rearrangement of MYC, bcl-6 and bcl-2
10.3760/cma.j.issn.0529-5807.2018.01.004
- VernacularTitle: 伴有MYC、bcl－2和bcl－6基因重排的高级别B细胞淋巴瘤的临床病理特征
- Author:
Jinying GONG
1
;
Yizhuo ZHANG
;
Jingdong ZHANG
;
Wei ZHANG
;
Jianqiang LI
;
Kun RU
;
Enbin LIU
Author Information
1. Department of Pathology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Tianjin 300020, China
- Publication Type:Journal Article
- Keywords:
Lymphoma, B-cell;
Gene rearrangement, B-lymphocyte;
In situ hybridization, fluorescence;
Prognosis
- From:
Chinese Journal of Pathology
2018;47(1):14-18
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinicopathologic features of patients with high-grade B-cell lymphomas (HGBL) that have rearrangements of MYC, bcl-6 and bcl-2.
Methods:One hundred and fifty-eight B-cell lymphomas patients from Institute of Hematology and Blood Diseases Hospital from January 2016 to April 2017 were detected by fluorescence in situ hybridization (FISH) with double color split-apart probes.
Results:Among 158 B-cell lymphomas, 3 cases with MYC, bcl-2 and bcl-6 rearrangements were identified, 1 of which also had CCND1/IgH translocation. All three patients were of older age, with poor prognostic parameters, multiple organs involvements, elevated LDH and advanced-tumor stage. Two of the three patients were treated with high-intensity chemotherapy and had no remission with an overall survival of 9 months and 11 months respectively. One patient had follow-up with no treatment. Histologically, all three cases showed a spectrum of morphologic features. Although initially categorized as lymphoblastic lymphoma, diffuse large lymphoma and mantle cell lymphoma respectively, two cases were associated with germinal center B-cell (GCB) immunophenotype and 1 case with non-GCB immunophenotype. They had a high proliferation index as assessed by immunostaining for Ki-67 (60%-90%).
Conclusions:MYC+ bcl-2+ bcl-6+ HGBL is an aggressive disease with multiple organ involvement, high serum LDH levels, advanced stage disease, poor prognosis and shorter patient survival. The diagnosis should be made by histopathology combined with FISH analysis. Its separation from other types of B cell large cell lymphoma is of clinical importance.
